| The jugulotympanic paragangliomas: 41 cases report.
Rev Laryngol Otol Rhinol (Bord).
2005;126(1):7-13.
OBJECTIVE: The
jugulotympanic paragangliomas (JTP) represents the most frequent
tumour of the middle ear but also of the temporal bone, after the
acoustic neurinoma. The management of these vascular tumours remains
uncleared. The purpose of this study was to report our experience
about JTP in the CHU of Grenoble. MATERIALS AND METHODS: Retrospective
study of 41 patients, between 1973 and 1996. Six stages A, 8 stages B
and 27 stages C are reported in whom 20 cases (49%) presented an
intracranial extension (classification of Fisch). There were 2
familial cases with multiple localisations, in particular carotid. All
the patients were divided in 3 groups: surgery or radiation therapy in
first intention, surgery followed by radiation therapy. RESULTS: A
total tumor removal without recurrence was achieved by surgery in more
than 95% of the cases with 6 years follow-up but was associated with
significant morbidity (major cranial nerve injury). We noticed one
death by laryngospasme (C2Di2 tumour operated by infratemporal A
approach). A stabilization of the tumour was obtained with
radiotherapy in first intention in 75% of the cases (5 years
follow-up) but with a risk of radionecrosis. A revision surgery was
necessary in 3 cases. CONCLUSION: The comparaison of our different
therapeutic management, surgery (23), radiation therapy (16) or
combined (2), encourage us to perform a radical surgery whenever
possible. Because of the slow rate of growth, the radiotherapy is
indicated for older patients, at risk for surgery or extensive tumors.
The objectives of the radiation therapy are to obtain a tumoral
stabilization with improvement of the symptoms and low morbidity. The
management of this rare pathology must be multidisciplinary. The
recent discoveries on genes encoding three succinate dehydrogenase
subunits (SDHD, SDHB et SDHC) will allow a genetic detection of
asymptomatic case and will define the procedures for their management,
coordinated by a national network PGL.NET. A retrospective study could
also study the real incidence of familial paragangliomas.
Jugulotympanic paraganglioma (glomus tumour)
presenting with recurrent epistaxis.J
Laryngol Otol. 2004 Feb;118(2):153-5.
A case is presented where a left jugulotympanic
paraganglioma (JTP) extended to the nasopharynx and the patient
presented with recurrent epistaxis. Although initial biopsy of an
aural polyp had been suggestive of the diagnosis several years
previously, the diagnosis was not confirmed until the patient
presented with recurrent epistaxis and severe anaemia. To the best of
our knowledge, this is the first case reported of such a presentation
of JTP.
Diagnosis and therapy of glomus tympanicum and glomus jugulare
tumors.Zhonghua
Er Bi Yan Hou Ke Za Zhi. 2004 Sep;39(9):543-5.
OBJECTIVE: To report on a series of patients with
glomus tympanicum or glomus jugulare tumors, and to focus on its
diagnosis, treatment, and outcomes. METHODS: Ten patients with glomus
tympanicum or glomus jugulare tumors at Peking Union College Hospital
during a 17-year period were reviewed retrospectively. RESULTS: There
were 7 patients with glomus tympanicum, 3 with glomus jugulare tumors,
1 patient with familial paraganglioma and 1 with functioning glomus
jugulare tumors. The most common presenting symptoms were pulsatile
tinnitus and hearing loss. Other symptoms included facial nerve
paralysis, vertigo, otalgia, dysphagia, hoarseness, throat sore,
episodic hypertension with headaches and tachycardia. The most common
physical sign was a vascular middle ear mass. The other physical signs
included Brown sign, upper neck mass, pharyngeal plump, Collet-Sicard
syndrome, Homer's syndrome as well as deficit of cranial nerve V. The
radiographic evaluation included computed tomograph (9 cases),
angiography (4 cases) and magnetic resonance imaging (1 case). Eight
patients initially refered to ENT department, and 2 patients initially
consulted neurology or endocrinology specialists. The treatment
included preoperative embolization in 2 cases, simple surgery in 4
cases and surgery followed by radiation therapy in 6 cases. No
significant complications occurred. Nine of the 10 patients were
followed up, but 1 was lost. The mean follow-up time was 12 years
(ranged, 2-19 years). No tumor recurrence occured in the 6 cases with
total tumor removal. Three cases with subtotal tumor resection had no
tumor progression. CONCLUSIONS: The diagnosis and treatment of glomus
tympanicum and glomus jugulare tumors is particularly challenging.
Typical clinical manifestations and radiographic evaluation should be
considered together to establish the diagnosis. The primary treatment
for glomus tympanicum is surgery, if necessary, followed by
radiotherapy. Subtotal tumor resection followed by radiation yields
satisfying outcome for glomus jugulare tumors.
Diagnosis and treatment of glomus jugulare tumor.Zhonghua
Yi Xue Za Zhi. 2002 Oct 25;82(20):1381-4.
OBJECTIVE: To investigate the clinical features,
diagnosis, and treatment of glomus jugulare tumor. METHODS: The data
of 37 patients of glomus jugulare tumor diagnosed by digital
subtraction angiography (DSA) and/or operation and pathology, 14 males
(37.8%) and 23 females (62.2%), with an average age of 37.2 (3.5
approximately 66 years) and an average course of 4.3 years (1 month -
19 years), were analyzed. RESULTS: The tumor was located in the left
ear in 19 cases, in the right ear in 17 cases, and in both ears in 1
case, totally 38 ears. The tumor was chromaffin in one case with
hypertension. One case was complicated by ipsilateral carotid body
tumor, and 2 cases were complicated by ipsilateral cholesteatoma.
Thirty cases (81.1%) presented pulsatile tinnitus and hearing loss as
the main symptoms. Thirteen cases (35.1%) were diagnosed as glomus
jugulare tumor at the first visit, and 24 cases (64.9%) were diagnosed
as other diseases with an average misdiagnosis period of 4.4 years.
Eighteen tumors originated from typanum and 28 tumors from glomus
jugulare. According the Fisch classification 1981, the tumors in 11
ears were type A, in 7 ears were type B, in 7 ears type C, in 5 ears
type D1, and in 8 ears type D2. All cases, except the one with
bilateral lesions, underwent operation. The tumor was completely
resected in 30 cases. Postoperatively, facial paralysis was seen in 6
cases, dizziness, hoarseness, and subauricular necrosis was seen in
one case respectively. Of the 25 patients followed up with a mean
follow-up time of 5.2 years (1.1 - 16.1 years), 3 died of lung cancer,
neuroblastoma, or extensive involvement of glomus jugulare tumor
respectively, 19 survived without tumor, and 3 survived with the
tumor. CONCLUSION: Glomus jugulare tumor is likely to involve
surrounding important tissues, and to be misdiagnosed. Early surgical
removal of the lesion is important.
Jugulotympanic glomus tumors: a report of 11 cases.
Zhonghua Er Bi Yan Hou Ke Za Zhi.
2000 Oct;35(5):348-51.
OBJECTIVE: To
evaluate the surgical technique in removal of jugulotympanic glomus
tumor. METHODS: Retrospective analysis of surgical techniques and
effects of the surgical treated 11 cases (1982-1998) of jugulotympanic
glomus tumors (tympanic type I, 1, II, 1, IV, 3; jugular type I, 1,
III, 5). RESULTS: The tumors of all 11 patients were removed
completely and the wounds healed smoothly. Only one case had
postoperative laryngeal nerve paralysis. There was no recurrence after
1-8 years follow-up. CONCLUSION: Suitable surgical techniques are
available for removal of jugulotympanic glomus tumors and are chosen
according to the size and location of the tumor. Postauricular
incision is suitable for Glomous tympanicum Type II, III, IV. Since
postauricular incision with superior and inferior extension provides a
good exposure of the neck and temporal bone, it is suitable for
surgical removal of Glomus jugulare type I. In case of Glomus jugulare
type III, postauricular large "C" incision may be chosen for resection
of the mid- and infra-temporal fosse, neck and skull base tumor.
Avascular
tympanojugular paraganglioma.Laryngoscope.
1996 Jun;106(6):721-3.
The avascular
paraganglioma described in this article appears to be the second such
tumor reported in the international literature and the first to be
reported in the tympanojugular region. Despite a highly suggestive
history and clinical appearance, the tumor showed no signs of
vascularization on radiologic studies. The pathologic postoperative
study confirmed the diagnosis of paraganglioma with extensive stromal
fibrosclerosis and without the typical well-vascularized thin fibrous
septa. In the authors' opinion, this observation is notable because of
the difficulties encountered in the correct diagnostic interpretation
of an avascular mass in the tympanojugular region. In such cases, the
possibility of a paraganglioma should always be considered.
Glomus jugulare tumors revisited: a ten-year
statistical follow-up of 231 cases.Laryngoscope.
1985 Mar;95(3):284-8.
Glomus jugulare tumors are rather common in the
middle ear and temporal bone. They are usually easy to diagnose and
surgically remove. However, there are a comparatively large number of
these growths which not only cause extensive local destruction, but
can spread distally and even have endocrine manifestations. This group
is difficult to diagnose and manage despite recent advances in
diagnostic procedures and the advent of skull base surgery. There is a
void in the literature of a large series of cases being followed for a
long period of time. This paper presents a comprehensive detailed
statistical ten-year follow-up of 231 glomus jugulare tumors. The
results suggest that our present diagnostic procedures often are
inadequate in finding small tumors, and our treatment and follow-up
are lacking because these tumors tend to recur often at a late date
and in a different form. A comprehensive method of approach of
diagnosis and treatment is described to manage all cases of glomus
jugulare.
Anatomy involved in the jugular foramen approach
for jugulotympanic paraganglioma resection.Neurosurg
Focus. 2004 Aug 15;17(2):E6.
The goal in paraganglioma resection is to allow
adequate exposure to remove the lesion while preserving cranial nerve
function. Knowledge of the anatomy of the jugular foramen is crucial
to this endeavor. In this report the authors describe a jugular
foramen approach for the resection of glomus jugulare tumors in cases
in which rerouting of the facial nerve can be avoided. This approach
provides adequate exposure of the jugular bulb for many jugulotympanic
paragangliomas without increased risk of injury to the facial nerve.
In addition, special circumstances surrounding intracranial and
carotid artery involvement are briefly discussed.
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